Huntington disease (HD) is the most frequent neurodegenerative disease caused by an expansion of glutamines repeats. The main clinical manifestations of HD are chorea, cognitive impairment and psychiatric disorders. The transmission of HD is autosomic dominant with a complete penetrance. The mutation responsible for HD, an unstable expansion of CAG repeat sequence, is located at the 5′ terminal part of the IT15 gene encoding the Huntingtin (htt). One important characteristic of HD is the vulnerability of a particular brain region, the striatum, despite similar expression of the mutated protein in other brain areas (Roze et al., 2008a). Furthermore, despite the early expression of mutated Htt (Exp-Htt) in all neuronal cells, ie as soon as birth, the first symptoms and neuropathological hallmarks appear at adulthood, around 40-45 years old. The age of onset of the disease is conversely proportional to the number of CAG repeats in the affected allele. Once the first symptoms have appeared, the disease progresses and leads progressively to death. One currently admitted hypothesis is that alteration of specific signalling pathways during ageing increases Exp-Htt-induced molecular alterations, specifically or primarily in striatal neurons. So far, there are no available therapies aimed at slowing down disease progression and consequently HD progresses inexorably to death. Thus, it is important to find strategies for therapy in HD.